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Schnitzler syndrome

MedGen UID:
141892
•Concept ID:
C0524988
•
Disease or Syndrome
Synonym: Schnitzler Syndrome
SNOMED CT: Schnitzler syndrome (402415001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018304
Orphanet: ORPHA37748

Definition

Schnitzler syndrome is a rare, underdiagnosed disorder in adults characterized by recurrent febrile rash, bone and/or joint pain, enlarged lymph nodes, fatigue, a monoclonal IgM component, leukocytosis and systemic inflammatory response. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSchnitzler syndrome
Follow this link to review classifications for Schnitzler syndrome in Orphanet.

Professional guidelines

PubMed

Schnitzler Syndrome: Insights into Its Pathogenesis, Clinical Manifestations, and Current Management.
Braud A, Lipsker D
Biomolecules 2024 May 31;14(6) doi: 10.3390/biom14060646. PMID: 38927050Free PMC Article
IgM monoclonal gammopathies of clinical significance: diagnosis and management.
Khwaja J, D'Sa S, Minnema MC, Kersten MJ, Wechalekar A, Vos JM
Haematologica 2022 Sep 1;107(9):2037-2050. doi: 10.3324/haematol.2022.280953. PMID: 35770530Free PMC Article
Efficacy and safety of canakinumab treatment in schnitzler syndrome: A systematic literature review.
Betrains A, Staels F, Vanderschueren S
Semin Arthritis Rheum 2020 Aug;50(4):636-642. Epub 2020 May 25 doi: 10.1016/j.semarthrit.2020.05.002. PMID: 32502728

Recent clinical studies

Etiology

Genetics of somatic auto-inflammatory disorders.
Poulter JA, Savic S
Semin Hematol 2021 Oct;58(4):212-217. Epub 2021 Oct 9 doi: 10.1053/j.seminhematol.2021.10.001. PMID: 34802542
Incomplete Schnitzler Syndrome.
Henning MAS, Jemec GBE, Ibler KS
Acta Dermatovenerol Croat 2020 Jul;28(1):38-40. PMID: 32650850
Schnitzler Syndrome: a Review.
Gusdorf L, Lipsker D
Curr Rheumatol Rep 2017 Aug;19(8):46. doi: 10.1007/s11926-017-0673-5. PMID: 28718061
Schnitzler Syndrome.
Badawi AH, Gierer S, Fraga GR
Allergy Asthma Proc 2014 Jan-Feb;35(1):75-7. doi: 10.2500/aap.2014.35.3703. PMID: 24433601
Monoclonal gammopathies and malignancies in patients with chronic urticaria.
Karakelides M, Monson KL, Volcheck GW, Weiler CR
Int J Dermatol 2006 Sep;45(9):1032-8. doi: 10.1111/j.1365-4632.2006.02982.x. PMID: 16961504

Diagnosis

Neutrophilic Urticarial Dermatosis.
Orakwue A, Bray J, Comfere N, Sokumbi O
Dermatol Clin 2024 Apr;42(2):219-229. Epub 2023 Sep 15 doi: 10.1016/j.det.2023.08.009. PMID: 38423683
Cutaneous manifestations of monoclonal gammopathy.
Claveau JS, Wetter DA, Kumar S
Blood Cancer J 2022 Apr 11;12(4):58. doi: 10.1038/s41408-022-00661-1. PMID: 35411042Free PMC Article
Incomplete Schnitzler Syndrome.
Henning MAS, Jemec GBE, Ibler KS
Acta Dermatovenerol Croat 2020 Jul;28(1):38-40. PMID: 32650850
Neutrophilic urticarial dermatosis: A review.
Gusdorf L, Lipsker D
Ann Dermatol Venereol 2018 Dec;145(12):735-740. Epub 2018 Sep 14 doi: 10.1016/j.annder.2018.06.010. PMID: 30224079
Schnitzler syndrome.
Soubrier M
Joint Bone Spine 2008 May;75(3):263-6. Epub 2008 Feb 22 doi: 10.1016/j.jbspin.2007.07.014. PMID: 18378180

Therapy

Schnitzler Syndrome after COVID-19 Vaccination.
Asako M, Matsunaga H, Oka K, Ueda S
Intern Med 2022 Aug 1;61(15):2397. Epub 2022 May 31 doi: 10.2169/internalmedicine.9598-22. PMID: 35650133Free PMC Article
Tocilizumab treatment in patients with Schnitzler syndrome: An open-label study.
Bonnekoh H, Frischbutter S, Roll S, Maurer M, Krause K
J Allergy Clin Immunol Pract 2021 Jun;9(6):2486-2489.e4. Epub 2021 Feb 2 doi: 10.1016/j.jaip.2021.01.024. PMID: 33545397
The systemic autoinflammatory disorders for dermatologists. Part 2: disease examples.
Oldham J, Lachmann HJ
Clin Exp Dermatol 2020 Dec;45(8):967-973. Epub 2020 Sep 3 doi: 10.1111/ced.14251. PMID: 32882069
Neutrophilic urticarial dermatosis: A review.
Gusdorf L, Lipsker D
Ann Dermatol Venereol 2018 Dec;145(12):735-740. Epub 2018 Sep 14 doi: 10.1016/j.annder.2018.06.010. PMID: 30224079
Biologic Therapy in the Treatment of Chronic Skin Disorders.
Fernandez JM, Fernandez AP, Lang DM
Immunol Allergy Clin North Am 2017 May;37(2):315-327. Epub 2017 Mar 1 doi: 10.1016/j.iac.2017.01.006. PMID: 28366479

Prognosis

A Man With Recurrent Fever, Episodic Rash, and Pain.
Aslam F, Wiedmeier JE, DiCaudo DJ
Arthritis Care Res (Hoboken) 2021 Aug;73(8):1106-1113. Epub 2021 Jul 14 doi: 10.1002/acr.24142. PMID: 31961498
Paraprotein associated syndrome treated successfully with chemotherapy.
Gurung R, Robertson C, Vickers MA
BMJ Case Rep 2020 Sep 9;13(9) doi: 10.1136/bcr-2019-232866. PMID: 32912879Free PMC Article
Schnitzler Syndrome: a Review.
Gusdorf L, Lipsker D
Curr Rheumatol Rep 2017 Aug;19(8):46. doi: 10.1007/s11926-017-0673-5. PMID: 28718061
Urticarial vasculitis and Schnitzler syndrome.
Zuberbier T, Maurer M
Immunol Allergy Clin North Am 2014 Feb;34(1):141-7. Epub 2013 Oct 25 doi: 10.1016/j.iac.2013.09.007. PMID: 24262694
Schnitzler's syndrome: 3-year radiological follow-up.
Bertrand A, Feydy A, Belmatoug N, Fantin B
Skeletal Radiol 2007 Feb;36(2):153-6. Epub 2006 Apr 7 doi: 10.1007/s00256-006-0093-5. PMID: 16602013

Clinical prediction guides

Neutrophilic epitheliotropism, proposed as an auto-inflammatory condition of neutrophilic urticarial dermatosis including Schnitzler syndrome, is also observed in Japanese cases.
Nakaizumi H, Kambe N, Irie H, Kaku Y, Fujimoto M, Yoshifuji H, Kazuma Y, Katagiri K, Kanekura T, Kabashima K
J Dermatol 2024 Apr;51(4):592-596. Epub 2023 Dec 6 doi: 10.1111/1346-8138.17067. PMID: 38057948Free PMC Article
A Man With Recurrent Fever, Episodic Rash, and Pain.
Aslam F, Wiedmeier JE, DiCaudo DJ
Arthritis Care Res (Hoboken) 2021 Aug;73(8):1106-1113. Epub 2021 Jul 14 doi: 10.1002/acr.24142. PMID: 31961498
Case Report: Extensive Phosphorylation of Interleukin-1 Receptor-Associated Kinase 4 in a Patient With Schnitzler Syndrome.
Hodl I, Bosch P, Dreo B, Stradner MH
Front Immunol 2020;11:576200. Epub 2020 Sep 30 doi: 10.3389/fimmu.2020.576200. PMID: 33123160Free PMC Article
Partial response to cyclosporine in a patient with Schnitzler's syndrome.
Carbone J, Paravisini A, Sarmiento E, Rodríguez-Molina J, Fernández-Cruz E
Allergol Immunopathol (Madr) 2007 Mar-Apr;35(2):71-3. doi: 10.1157/13101341. PMID: 17428403
Report of a case of Schnitzler's syndrome treated successfully with interferon alpha 2b.
Schartz NE, Buder S, Sperl H, Audring H, Paus R, Tebbe B, Krüger K, Sterry W
Dermatology 2002;205(1):54-6. doi: 10.1159/000063139. PMID: 12145435

Recent systematic reviews

A Chinese case series of Schnitzler syndrome and complete remission in one tocilizumab-treated patient.
Yan R, Cao W, Liu X, Li F, Shen M
Clin Rheumatol 2020 Dec;39(12):3847-3852. Epub 2020 Jun 9 doi: 10.1007/s10067-020-05204-2. PMID: 32519052
Efficacy and safety of canakinumab treatment in schnitzler syndrome: A systematic literature review.
Betrains A, Staels F, Vanderschueren S
Semin Arthritis Rheum 2020 Aug;50(4):636-642. Epub 2020 May 25 doi: 10.1016/j.semarthrit.2020.05.002. PMID: 32502728

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